Male Speaker: Most children begin life with an immune system that learns to recognize and destroy hostile germs. But in 1952, an army doctor named Colonel Ogden Bruton made a startling discovery he found something missing in the blood of one young patient.
In people with a healthy immune system, white blood cells are constantly on guard. Some attack bacteria and viruses directly. Others produce antibodies, proteins that bind with specific germs and attract the body's defenses.
Colonel Bruton's patient could not make antibodies and this left him vulnerable to even the most common germs. It was the first known case of primary immunodeficiency.
Dr. Charlotte Cunningham-Rundles: Primary immunodeficiency is an immune defect that a person is born with and that's distinguished from an immune defect which might be caused by a virus or perhaps an immune defect that might be caused by chemotherapy or cancer or some other external cause.
Male Speaker: Several hundred children are born with primary immunodeficiency every year, among them, one-year-old twins Tyler and Connor Love. When they were two months old, tests showed they were barely producing any antibodies.
Jodie Love: Their numbers were so low that they were at risk not only for not only upper respiratory infections but other infections that could have been real bad. That could have actually have killed them.
Dr. Melvin Berger: One way we can help those patients is by taking the antibodies that normal people have made, for example in response to the vaccines that they got, and purify those antibodies and then give them to the patient who can't make antibodies.
Male Speaker: The process begins when healthy donors provide plasma, the part of the blood that contains antibody-rich immunoglobulin. A procedure known as plasmapheresis is used to collect the plasma while returning other blood cells to the donor. The plasma then undergoes extremely sensitive testing to check for viruses or other infectious agents.
Dr. Charlotte Cunningham-Rundles: Every single product that comes out of blood or plasma is very highly supervised not only in the collection procedures but also in the subsequent manufacturing procedures.
Male Speaker: Plasma that tests germ-free is frozen and shipped to a facility for processing under highly sterile conditions. As the individual plasma units are pooled together, robotic machinery helps minimize contact with human hands.
The plasma undergoes multiple steps to pull out unwanted proteins and inactivate any viruses that may have missed detection.
The end result is a solution of purified immunoglobulin or IgG that contains antibodies from many different donors. It can be given intravenously once every three or four weeks or patients can use small needles and a pump to infuse at home once a week.
Dr. Charlotte Cunningham-Rundles: You can actually give the amount of gamma globulin that's really required to bring that person's IgG level up to, into the normal range or if you want to, even higher than the normal range. So that's a wonderful accomplishment that I think we're all thrilled about.
Dr. Melvin Berger: It's really changed patients' lives in the time I've been in practice.
Jodie Love: They might have to miss a slumber party or a birthday party if someone has a cold or someone has the flu. As long as everybody is healthy around them, they should be able to do what every other child does.
Male Speaker: Despite effective therapies, primary immunodeficiency often goes undetected and many patients don't receive treatment until they have been chronically ill. The ultimate goal is to have all babies with serious immune defects begin treatment before they get their first infection.
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